1 40 5 Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource July 2023 List Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month July List 2023 URL Address <a href="http://doi.org/10.2144/fsoa-2022-0062" target="_blank" rel="noreferrer noopener"> http://doi.org/10.2144/fsoa-2022-0062</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Neonatal intensive care unit nurses knowledge and attitude toward neonatal palliative care: review of the literature Publisher An entity responsible for making the resource available Future Science Open Access Date A point or period of time associated with an event in the lifecycle of the resource 2023 Subject The topic of the resource attitude; Infant Newborn; Intensive Care Units; Intensive Care Units Neonatal; knowledge; Npc; nurse; Palliative Care Creator An entity primarily responsible for making the resource Abuhammad S; Elayyan M; Ababneh H Description An account of the resource AIM: To review studies regarding neonatal nurses' knowledge and attitude toward neonatal palliative care (NPC). METHOD: The researchers searched internet sources such as Google Scholar for NPC, Nurses, Knowledge, Attitude, and Educational Intervention. RESULTS: Subheadings identified in the literature review were Nurses Knowledge toward NPC in NICU, Nurses Attitude toward NPC in NICU, correlation between Knowledge and Attitude toward NPC in NICU, The Effect of Educational program on Nurses Knowledge and Attitude toward NPC in NICU, and the Predictors of Knowledge and Attitude toward NPC among Nurses in NICU and Barriers to NPC provision and improvement. CONCLUSION: There are few studies from different nations regarding NPC found nurses have an inadequate knowledge of NPC, which also reflects their attitude. Objective: To review studies regarding neonatal nurses' information and behavior toward end-of-life care. Method: The researcher searched internet sources such as Google Scholar, PubMed, Medline, and ResearchGate for end of life, Nurses, Information, Behavior, And Educational Program. Results: Subheadings identified in the literature review were nurses information toward end of life care in neonate care unit, nurses attitude toward end of life in neonate care unit, correlation between knowledge and attitude toward end of life in neonate care unit, the effect of educational program on nurses information and behavior toward end of life in neonate care and the predictors of information and behavior toward end of life among nurses in neonate and barriers to end of life. Conclusion: There are few studies from different nations regarding end of life found nurses have an inadequate information of end of life, which also reflects their behaviors. eng Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.2144/fsoa-2022-0062" target="_blank" rel="noreferrer noopener">10.2144/fsoa-2022-0062</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). 2023 Ababneh H Abuhammad S Attitude Elayyan M Future Science Open Access Infant Newborn Intensive Care Units Intensive Care Units Neonatal July List 2023 Knowledge NPC Nurse Palliative Care Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. URL Address <a href="http://doi.org/10.1016/j.ijporl.2015.11.009" target="_blank" rel="noreferrer noopener">http://doi.org/​10.1016/j.ijporl.2015.11.009</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Otorhinolaryngological, Audiovestibular and swallowing manifestations of patients with Niemann-Pick disease Type C Publisher An entity responsible for making the resource available International Journal of Pediatric Otorhinolaryngology Date A point or period of time associated with an event in the lifecycle of the resource 2016 Subject The topic of the resource adolescent; hearing; dysphagia; priority journal; school child; preschool child; prospective study; human; article; child; female; male; controlled study; clinical article; auditory response; Balance disorders; body equilibrium; endoscopy; Flexible endoscopy; hearing impairment; Hearing problems; Niemann Pick disease/di [Diagnosis]; Niemann-Pick disease; otorhinolaryngology; pure tone audiometry; stabilography; swallowing; Swallowing disorders; vestibular system; feeding difficulties; tone and motor problems; NPC; penetration; aspiration scale; trajectory; characteristics; postural imbalance; dysphagia Creator An entity primarily responsible for making the resource Senirli R T; Kuscu O; Akyol U; Topcu M; Yigit O; Aksoy S; Demir N Description An account of the resource Objectives: The aim of this study is to evaluate audiovestibular and swallowing impairment of patients with NPC. Methods: Audiovestibular and swallowing evaluation were performed on patients with Niemann-Pick disease type C (NPC) at Hacettepe University between 20013 and 2015 prospectively. Pure-tone audiometry (PTA), Auditory Brain stem response (ABR), Flexible endoscopic evaluation of swallowing (FEES) test and posturography were done. Hearing, swallowing and balance states were measured. Results: There were 16 patients (5 male and 11 female, with a median age of 6.5 years old). The most common ABR abnormalities observed were absent waves I and III (%70 absent I waves, %43.75 absent III waves). Twelve of sixteen patients (%75) had an ABR abnormality in at least one ear, of these, four patients had normal hearing and three of them had periferal hearing loss. 12 (75%) patients had complaint of postural imbalance. 11(69%) of patients had peripheral and one (6%) patient had central impairment. Nine of sixteen patients (56.25%) show some degree of dysphagia (either penetration or aspiration). Two patients (12.5%) showed aspiration both liquid and viscous nutrition. Three patients (18.75%) showed aspiration primarily in liquids and two of them had penetration with viscous nutrition. Three patients (18.75%) had penetration with no aspiration neither liquid nor viscous nutrition (PEN-ASP score was 3, 3, 5, respectively). Conclusion: There is no curative treatment for this devastating and fatal disorder and hearing impairment, balance and swallowing disorders can be seen especially late onset form of disease. Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1016/j.ijporl.2015.11.009" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2015.11.009</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). 2016 Adolescent Aksoy S Akyol U Article aspiration scale auditory response Balance disorders body equilibrium characteristics Child Clinical Article Controlled Study Demir N Dysphagia Endoscopy feeding difficulties Female Flexible endoscopy hearing hearing impairment Hearing problems Human International Journal of Pediatric Otorhinolaryngology Kuscu O Male Niemann Pick disease/di [Diagnosis] Niemann-Pick Disease NPC Otorhinolaryngology penetration postural imbalance Preschool Child Priority Journal Prospective Study pure tone audiometry School Child Senirli R T stabilography Swallowing Swallowing disorders tone and motor problems Topcu M Trajectory vestibular system Yigit O Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. URL Address <a href="http://doi.org/10.1177/0883073817741054" target="_blank" rel="noreferrer noopener">http://doi.org/​10.1177/0883073817741054</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Gait, Balance, and Coordination Impairments in Niemann Pick Disease, Type C1 Publisher An entity responsible for making the resource available Journal of Child Neurology Date A point or period of time associated with an event in the lifecycle of the resource 2018 Subject The topic of the resource adolescent; retrospective study; priority journal; scoring system; school child; outcome assessment; preschool child; intervention study; human; article; child; female; male; adult; clinical article; young adult; comparative study; body equilibrium; stabilography; performance; neurologic examination; balance impairment; coordination disorder; gait disorder; Niemann Pick disease; 2 hydroxypropyl beta cyclodextrin; 94035-02-6 (2 hydroxypropyl beta cyclodextrin); case control study; clinical evaluation; disease severity assessment; human development; molecular stability; motion analysis system; NIH NPC Neurologic Severity Scale; research; spatiotemporal analysis; upper limb; vts 270; tone and motor problems; NPC; trajectory; characteristics Creator An entity primarily responsible for making the resource Sansare A; Zampieri C; Alter K; Stanley C; Farhat N; Keener L A; Porter F Description An account of the resource This is the first study to objectively measure gait, balance, and upper limb coordination in a group of patients with NPC1 and compare the results to age and gender matched controls. This is also the first study to report effect sizes in these measures. Spatiotemporal gait analysis, static and dynamic posturography, and upper limb reaching motion analysis were performed. The findings showed that the NPC1 subjects had statistically significant deficits on 12 out of the 16 parameters investigated compared to controls, and large effect sizes for all but 1 parameter. When ranking the variables in terms of the effect sizes, the top 5 included at least 1 parameter from each of the 3 motor domains investigated. These results can provide insight to clinical researchers on the selection of outcome measures for longitudinal and interventional studies. Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1177/0883073817741054" target="_blank" rel="noreferrer noopener">10.1177/0883073817741054</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). 2 hydroxypropyl beta cyclodextrin 2018 94035-02-6 (2 hydroxypropyl beta cyclodextrin) Adolescent Adult Alter K Article balance impairment body equilibrium case control study characteristics Child Clinical Article clinical evaluation Comparative Study coordination disorder disease severity assessment Farhat N Female gait disorder Human Human Development intervention study Journal of Child Neurology Keener L A Male molecular stability motion analysis system Neurologic Examination Niemann Pick disease NIH NPC Neurologic Severity Scale NPC outcome assessment performance Porter F Preschool Child Priority Journal Research Retrospective Study Sansare A School Child scoring system spatiotemporal analysis stabilography Stanley C tone and motor problems Trajectory Upper limb vts 270 Young Adult Zampieri C Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. URL Address <a href="http://doi.org/10.1016/j.jns.2006.05.054" target="_blank" rel="noreferrer noopener">http://doi.org/​10.1016/j.jns.2006.05.054</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Niemann-Pick C disease in Spain: clinical spectrum and development of a disability scale Publisher An entity responsible for making the resource available Journal of the Neurological Sciences Date A point or period of time associated with an event in the lifecycle of the resource 2006 Subject The topic of the resource Adolescent; Adult; Age of Onset; Carrier Proteins/ge [Genetics]; Cerebellar Ataxia/di [Diagnosis]; Cerebellar Ataxia/ep [Epidemiology]; Cerebellar Diseases/di [Diagnosis]; Cerebellar Diseases/ep [Epidemiology]; Child; Preschool; Comorbidity; Disability; Evaluation; Dysarthria/di [Diagnosis]; Dysarthria/ep [Epidemiology]; Female; Genetic Predisposition to Disease/ge [Genetics]; Infant; feeding difficulties; sleep disturbance; tone and motor problems; NPC; tool development; scale development Creator An entity primarily responsible for making the resource Iturriaga C; Pineda M; Fernandez-Valero E M; Vanier M T; Coll M J Description An account of the resource OBJECTIVES: To describe the clinical evolution of Niemann-Pick C disease to identify possible factors involved in the diagnosis and severity of the disease. METHODS: A clinical database and a severity scale was created to evaluate 45 patients diagnosed with Niemann-Pick type C in the last 28 years in Spain. RESULTS: Complete clinical data were obtained from 30 patients, all were confirmed to have mutations in the NPC1 gene. Regarding clinical form, 3 were perinatal, 7 severe infantile, 6 late infantile, 11 juvenile and 3 adult. Biochemical phenotype was classic in 26. Splenomegaly was present in 28 patients (93%) with a wide range of age at detection. The first symptom of neurological disease was clumsiness, followed in 2-4 years by cerebellar signs. Ophthalmoplegia appeared 2-4 years later and became complete 1-2 years after onset. Dysarthria appeared by the time of complete ophthalmoplegia. Diagnosis was made before the onset of neurological signs in patients with the severe infantile form, at the time of onset of cerebellar signs in the late infantile form and complete ophthalmoplegia in late onset forms. CONCLUSIONS: In our series, splenomegaly is present in 96% of patients, even in late onset forms during the first years of life. Clumsiness in children with otherwise normal motor development precedes the onset of ataxia by 2-4 years in Niemann Pick type C. A disability scale could be useful for monitoring evolution, establishing possible phenotypic correlations and evaluating future therapies. Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1016/j.jns.2006.05.054" target="_blank" rel="noreferrer noopener">10.1016/j.jns.2006.05.054</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). 2006 Adolescent Adult Age of Onset Carrier Proteins/ge [Genetics] cerebellar ataxia/di [Diagnosis] Cerebellar Ataxia/ep [Epidemiology] Cerebellar Diseases/di [Diagnosis] Cerebellar Diseases/ep [Epidemiology] Child Coll M J Comorbidity Disability Dysarthria/di [Diagnosis] Dysarthria/ep [Epidemiology] Evaluation feeding difficulties Female Fernandez-Valero E M Genetic Predisposition to Disease/ge [Genetics] Infant Iturriaga C Journal Of The Neurological Sciences NPC Pineda M Preschool scale development sleep disturbance tone and motor problems tool development Vanier M T Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. URL Address <a href="http://doi.org/10.1016/j.ejpn.2014.07.009" target="_blank" rel="noreferrer noopener">http://doi.org/​10.1016/j.ejpn.2014.07.009</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Successful treatment of cataplexy in patients with early-infantile Niemann-Pick disease type C: use of tricyclic antidepressants Publisher An entity responsible for making the resource available European Journal of Paediatric Neurology Date A point or period of time associated with an event in the lifecycle of the resource 2014 Subject The topic of the resource tone and motor problems; NPC; pharmacologic intervention; imipramine; cataplexy Creator An entity primarily responsible for making the resource Cak H T; Haliloglu G; Duzgun G; Yuce A; Topcu M Description An account of the resource Cataplexy is a brief episode of bilateral loss of muscle tone with intact consciousness, triggered by a variety of strong emotions such as anger, laugh, humor or surprise and it is considered to represent the physiologic atonia of rapid eye movement sleep. On the other hand, Niemann-Pick type C is a neurodegenerative lysosomal storage disease, characterized by the accumulation of cholesterol and glycosphingolipids. Cataplexy is a relatively specific and common neurologic sign seen in almost 50% of all patients with Niemann-Pick type C. The aim of this report is to demonstrate the successful treatment of cataplexy with the use of a tricyclic antidepressant imipiramine, in two patients between the ages 6-9, with mild to moderate mental retardation, molecularly diagnosed as Niemann-Pick type C 1 and currently under miglustat treatment and to discuss the possible mechanisms of drug action in the light of cataplexy and Niemann-Pick type C pathophysiology.Copyright © 2014 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved. Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1016/j.ejpn.2014.07.009" target="_blank" rel="noreferrer noopener">10.1016/j.ejpn.2014.07.009</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). 2014 Cak H T cataplexy Duzgun G European Journal of Paediatric Neurology Haliloglu G Imipramine NPC pharmacologic intervention tone and motor problems Topcu M Yuce A